CTICasesCongenital Heart Disease with Hypoplastic Aortic Arch and Diffuse Iliac Artery Hypoplasia
Imaging findings
A full-term infant transferred for congenital heart disease had an enlarged pulmonary artery with a hypoplastic, three-vessel left aortic arch of normal branching, a small high muscular ventricular septal defect, and a large secundum atrial septal defect with right heart enlargement, all confirmed on echocardiography. After the ductus arteriosus closed, the lower extremities became underperfused; there was no blood pressure gradient between upper and lower extremities, but angiography demonstrated diffusely hypoplastic, smooth common iliac arteries extending to their bifurcation without luminal irregularity, as well as a similarly hypoplastic right subclavian and axillary-to-arm arterial segment.
Key takeaways
This distribution of diffuse iliac artery hypoplasia without luminal irregularity is an extremely rare cause of lower extremity hypoperfusion, with only isolated case reports in the literature. Congenital rubella syndrome was considered but not supported by the clinical history, and the etiology of the iliac hypoplasia, distinct from the hypoplastic arch explained by the underlying congenital heart disease, remained unclear. Because the iliac arteries were too small for standard arterial access, catheterization was planned via a transvenous route across the atrial septal defect.
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